Journal of IMAB - Annual Proceeding (Scientific Papers) · Published 2026-07-13 · DOI 10.5272/jimab.2026323.6964
Margarita Pironeva, Kosta Kostov
Polymyositis is an idiopathic inflammatory myopathy classified under a heterogeneous group of autoimmune diseases predominantly presenting with symmetrical proximal muscle weakness, weakness of neck muscles with the manifestation of a "dropped head" symptom, dysphagia, dysphonia; elevated serum levels of creatine kinase (CK), sometimes in conjunction with elevated liver enzymes; characteristic EMG findings and muscle biopsy. The presence of myositis-specific antibodies in more than half of the patients facilitates the definition of distinct clinical subtypes and aids in accurate diagnosis, with an associated higher risk of involvement of other organs and systems such as the lungs, heart, liver, etc., most commonly presenting as interstitial pulmonary fibrosis, cardiomyopathy, and significantly less frequently primary biliary cirrhosis. A twofold increase in malignancies has been established compared to the general population, or the manifestation of myositis can be presented as a paraneoplastic syndrome. The first-line treatment method is the administration of intravenous corticosteroids in moderate or high doses combined with cytostatic therapy, with rare necessity for intravenous immunoglobulin administration, while plasmapheresis is considered an inapplicable method. We report a case of polymyositis with characteristic clinical and serological findings, with severe respiratory failure, developing twice in the absence of interstitial pulmonary fibrosis, primarily secondary myocardial damage during disease activity, and the presence of asymptomatic ovarian cancer and primary biliary cirrhosis discovered during the course of the disease, demonstrating the best therapeutic effectiveness from plasmapheresis. Accurate diagnosis relies on clinical, electrophysiological, and immunological findings, posing diagnostic challenges and questions that require the collaborative efforts of a multidisciplinary team.
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Pironeva, M., Kostov, K. (2026). POLYMYOSITIS ASSOCIATED WITH PRIMARY BILIARY CIRRHOSIS AND OVARIAN CARCINOMA – CLINICAL CASE PRESENTATION AND LITERATURE REVIEW. Journal of IMAB - Annual Proceeding (Scientific Papers). https://doi.org/10.5272/jimab.2026323.6964